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The intermuscular 3-7 Hz drive is not affected by distal proprioceptive input in myoclonus-dystonia

Journal

Experimental Brain Research

Volume | Issue number

202 | 3

Pages (from-to)

681-691

Document type

Article

Faculty

Faculty of Medicine (AMC-UvA)

Abstract

In dystonia, both sensory malfunctioning and an abnormal intermuscular low-frequency drive of 3-7 Hz have been found, although
cause and effect are unknown. It is hypothesized that sensory processing is primarily disturbed and induces this drive. Accordingly,
experimenter-controlled sensory input should be able to influence the frequency of the drive. In six genetically confirmed
myoclonus-dystonia (MD) patients and six matched controls, the low-frequency drive was studied with intermuscular coherence
analysis. External perturbations were applied mechanically to the wrist joint in small frequency bands (0-4, 4-8 and 8-12
Hz; 'angle protocol) and at single frequencies (1, 5, 7 and 9 Hz; 'torque' protocol). The low-frequency drive was found in
the neck muscles of 4 MD patients. In these patients, its frequency did not shift due to the perturbation. In the torque protocol,
the externally applied frequencies could be detected in all controls and in the two patients without the common drive. The
common low-frequency drive was not be affected by external perturbations in MD patients. Furthermore, the torque protocol
did not induce intermuscular coherences at the applied frequencies in these patients, as was the case in healthy controls
and in patients without the drive. This suggests that the dystonic 3-7 Hz drive is caused by a sensory-independent motor drive
and sensory malfunctioning in MD might rather be a consequence than a cause of dystonia

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